Linask Kersti K, Han Ming-Da, Linask Kaari L, Schlange Thomas, Brand Thomas
Department of Cell Biology, University of Medicine and Dentistry of New Jersey-SOM, Stratford, New Jersey, USA.
Dev Dyn. 2003 Oct;228(2):217-30. doi: 10.1002/dvdy.10383.
Dextral looping of the heart is regulated on multiple levels. In humans, mutations of the genes CFC and Pitx2/RIEG result in laterality-associated cardiac anomalies. In animal models, a common read-out after the misexpression of laterality genes is heart looping direction. Missing in these studies is how laterality genes impact on downstream morphogenetic processes to coordinate heart looping. Previously, we showed that Pitx2 indirectly regulates flectin protein by regulating the timing of flectin expression in one heart field versus the other (Linask et al. [2002] Dev. Biol. 246:407-417). To address this question further we used a reported loss-of-function approach to interfere with chick CFC expression (Schlange et al. [2001] Dev. Biol. 234:376-389) and assaying for flectin expression during looping. Antisense CFC treatment results in abnormal heart looping or no looping. Our results show that regardless of the sidedness of downstream Pitx2 expression, it is the sidedness of predominant flectin protein expression in the extracellular matrix of the dorsal mesocardial folds and splanchnic mesoderm apposed to the foregut wall that is associated directly with looping direction. Thus, Pitx2 can be experimentally uncoupled from heart looping. The flectin asymmetry continues to be maintained in the secondary heart field during looping.
心脏的右旋环化在多个层面上受到调控。在人类中,CFC基因和Pitx2/RIEG基因的突变会导致与左右侧相关的心脏异常。在动物模型中,左右侧基因错误表达后的一个常见观察指标是心脏环化方向。这些研究中缺失的是左右侧基因如何影响下游形态发生过程以协调心脏环化。此前,我们表明Pitx2通过调节flectin蛋白在一个心脏区域相对于另一个心脏区域的表达时间来间接调节flectin蛋白(Linask等人,[2002]《发育生物学》246:407 - 417)。为了进一步解决这个问题,我们采用了一种已报道的功能丧失方法来干扰鸡的CFC表达(Schlange等人,[2001]《发育生物学》234:376 - 389),并在环化过程中检测flectin的表达。反义CFC处理会导致心脏环化异常或无环化。我们的结果表明,无论下游Pitx2表达的左右侧性如何,与环化方向直接相关的是背侧心肌褶皱和与前肠壁相邻的脏壁中胚层细胞外基质中主要flectin蛋白表达的左右侧性。因此,Pitx2在实验中可以与心脏环化解耦。在环化过程中,flectin的不对称性在次级心脏区域持续保持。